Dear Doctor Column, February 14, 2005

Behcet's Causes Painful Sores; Is Rare in U.S.

Question:

I am a 39-year-old African American female with a diagnosis of Behçet's disease. I have constant leg and back pain as if my bones are breaking and the fatigue, along with depression, has been difficult. What can you tell me about the disease? My primary care physician has referred me to a rheumatologist.

Answer:

Behçet's disease is a chronic condition that causes very painful sores or ulcers in the mouth and on the genitals and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints) and inflammation of the digestive tract, brain, and spinal cord. Behçet's disease, named for the Turkish dermatologist who first described it, is common in the Middle East, Asia, and Japan, but is rare in the U.S. Usually beginning in an individual's 20s or 30s, the disease affects men more often than women.

The exact cause of Behçet's disease is unknown. Most of the symptoms are caused by inflammation of the blood vessels. Inflammation is the body's reaction to injury or disease and is marked by swelling, redness, heat, and pain. Doctors think an autoimmune reaction may cause blood vessels to become inflamed. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues.

Researchers believe two factors are important in the development of Behçet's disease: First, that abnormalities of the immune system, probably inherited, make some people susceptible to the disease; second, that something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people. For instance, researchers have found that people who have frequent strep infections (caused by Streptococcus bacteria) are more likely to develop the disease.

Behçet's disease affects each person differently. Some people have only mild symptoms, such as skin sores or ulcers in the mouth or on the genitals. Others have more severe disease, such as meningitis or inflammation of the membranes that cover the brain and spinal cord.

Diagnosing Behçet's disease is difficult because there is no specific test for it. Less than half of the individuals initially thought to have Behçet's disease actually have it. The doctor must examine a patient with symptoms and rule out other conditions with similar signs symptoms. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time.

Although there is no cure for Behçet's disease, the symptoms can usually be controlled with medication, rest, and exercise. Treatment goals are to reduce discomfort and prevent serious complications, such as disability from arthritis or blindness from the eye inflammation. A rheumatologist (a doctor specializing in arthritis) often manages the patient's overall treatment and treats joint disease.

The type of medicine and the length of treatment depend on the person's symptoms and how severe their symptoms are. It is likely that a combination of treatments will be needed to relieve specific symptoms.

Scientists are exploring possible genetic, bacterial, and viral causes of Behçet's disease, as well as improved drug treatment. Researchers are also investigating factors in the environment, such as a bacterium or virus, that could trigger the disease. For example, researchers suspect that herpes virus type 1, a virus that causes cold sores, also may be associated with the disease in susceptible people.

For more information, visit the American Behçet's Disease Association Web site.